Adult diagnosis of Swyer-James-MacLeod syndrome: a case report

Abstract

IntroductionSwyer-James-MacLeod syndrome or unilateral hyperlucent lung syndrome is a rare entity associated with postinfectious bronchiolitis obliterans occurring in childhood. It is characterized by hypoplasia and/or agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion.Case presentationHere we report the case of a 53-year-old Caucasian woman with Swyer-James-MacLeod syndrome found in the differential diagnosis workup for a new onset of heart failure, secondary to pulmonary arterial hypertension complicated by a patent ductus arteriosus.ConclusionTypically, this disorder is diagnosed in childhood after evaluation for recurrent respiratory infections, but sometimes an indolent course means diagnosis is not made until adulthood.

Adult Swyer-James-MacLeod syndrome: report of two cases and review of the literature

“…It may remain misdiagnosed till adulthood when the diagnosis may be made incidentally or sometimes with very important symptoms associated with defects of the pulmonary function test. 3 In these patients the diagnosis of SJMS entails the exclusion of other causes of unilateral hypertranslucency of the lung, such as lung bullae, cysts or congenital vascular defects or pulmonary embolism. We present two cases of adult patients for whom the diagnosis of the SJML syndrome was made in the course of the workup for dyspnea and in which volumetric high resolution computed tomography (v-HRTC) with post processing to maximum intensity projection (MIP) clearly demonstrated the hypoplastic pulmonary vascular defects.…”

An uncommon obliterative lung disease: Swyer–James–MacLeod syndrome

“…This syndrome is most often seen in children, but can rarely present in adults with a past medical history of recurrent respiratory infections. SJMS can be caused by numerous microbes including adenovirus types 3,7, and 21, paramyxovirus morbillivirus, Bordetella pertussis, Mycoplasma pneumoniae, Mycobacterium tuberculosis, and influenza A [2,3]. These infections can cause post-infectious bronchiolitis obliterans, and may also cause hypoplasia or agenesis of the pulmonary vasculature.…”

“…These infections can cause post-infectious bronchiolitis obliterans, and may also cause hypoplasia or agenesis of the pulmonary vasculature. In the latter scenario, hypoperfusion of the pulmonary parenchyma will result in unilateral hyperlucency of the lung on radiographic images [2,3]. The symptoms of SJMS can vary from asymptomatic to chronic or recurring lung infections, dyspnea, or hemoptysis [1].…”

“…Unilateral lung hypoplasia, destruction of the pulmonary parenchyma and air trapping (bronchiectasis) are also likely to be visualized on CT imaging [1,2]. Abnormal pulmonary arterial vasculature characterized by decreased caliber may be visualized if CTA of the pulmonary vasculature is performed [2].…”

“…The treatment of SJMS is often conservative, and typically involves managing current, and preventing future pulmonary infections. The administration of the influenza and pneumococcal vaccines is, therefore, recommended [2]. Surgical consideration such as pneumonectomy, lobectomy, or segmentectomy has been documented to improve lung function and quality of life in patients who suffer from uncontrolled infections or severe lung damage [4].…”